Nephrology- Pastest

1. You are asked by your orthopaedic colleagues to review a 28-year-old victim of blunt trauma after a motorcycle accident. He has suffered extensive lower limb damage and requires large amounts of analgesia. The orthopaedic surgeons are concerned about his blood results, his potassium some hours after the accident is 6.7 mmol/l, calcium is 2.05 mmol/l, urine is positive to dipstick testing for blood. What diagnosis fits best with this clinical picture? Acute sepsis Hypovolaemia leading to pre-renal failure Rhabdomyolysis Your answer Direct renal trauma with perinephric haematoma Analgesic nephropathy Rhabdomyolysis occurs after an extensive blunt trauma, such as that occurring after a motorcycle accident. Diagnosis is made by the presence of myoglobin on urine dipstick (shows as haematuria), raised serum creatine kinase (CK) levels, hyperkalaemia, hypocalcaemia, hyperphosphataemia and hyperuricaemia. Aggressive iv fluid replacement is required to prevent acute renal failure, which may occur in up to 30% of cases of rhabdomyolysis. The rise in CK levels is detectable a few hours after injury and peaks at the 48-h stage. Rhabdomyolysis is also common after electrical injury, compartment syndrome, prolonged limb or tourniquet anaesthesia, extensive surgical dissection and infectious or inflammatory myopathies.

2. A 30-year-old man presents with frank haematuria and haemoptysis. A blood test shows microcytic hypochromic anaemia. Chest X-ray reveals bilateral infiltrates in the lower zones. What is the most likely diagnosis? Renal cell carcinoma Renal calculus Bronchial carcinoma Renal tuberculosis Goodpastures syndrome Your answer This man most probably has Goodpastures syndrome. The disease often starts with an upper respiratory tract infection followed by cough and intermittent haemoptysis, tiredness and eventually anaemia. Chest X-ray shadows are usually due to intrapulmonary haemorrhage. These features usually precede the development of an acute glomerulonephritis by several

weeks or months. Renal cell carcinoma rarely presents before the age of 40 years, the average age of presentation being 55 years. Patients present with a classic triad of haematuria, loin pain and a mass in the flank. Malaise, anorexia and weight loss may occur, and one-third of patients have hypertension. Bony metastases are common in bronchial carcinoma. There is frequent involvement of the liver. Although deposits are frequently found in the adrenal glands, the kidneys are seldom involved. Tuberculosis of the urinary tract may present with frequency, dysuria and/or haematuria. Adult postprimary pulmonary tuberculosis presents with a gradual onset of tiredness, malaise, anorexia, loss of weight, fever and cough. Sputum may be mucoid, purulent or bloodstained. The chest X-ray typically shows patchy or nodular shadows in the upper zones, loss of volume and fibrosis, with or without cavitation. Calcification may be present.

3. A 65-year-old man presents with haematuria, right loin pain and night sweats. Physical examination reveals a mass in the right flank. Blood tests show normocytic normochromic anaemia. What is the most likely diagnosis? Renal tract calculi Adult polycystic kidney disease Renal carcinoma Your answer Renal amyloidosis Chronic pyelonephritis The average age of presentation of renal cell carcinoma is around 55 years. Patients present with a classic triad of haematuria, loin pain and a mass in the flank. Malaise, anorexia and weight loss may occur and one-third of patients has hypertension. Pain in ureteric calculus, which is most often associated with haematuria, is extremely severe. Night sweats do not occur in this condition. Renal amyloidosis presents with asymptomatic proteinuria, nephritic syndrome or renal failure. Haematuria is not a feature. Chronic pyelonephritis is also known as reflux nephropathy. This starts in infancy or early childhood, predisposes to recurrent infections and progressive renal fibrosis and loss of function. The kidneys are small, shrunken and scarred. Adult (autosomal-dominant) polycystic kidney disease is an inherited disorder usually presenting in adult life. It is mostly bilateral and presents with acute loin pain and/or haematuria. Night sweats are not characteristic of this disease. 4. A 27-year-old woman is in end-stage renal disease. She has been started on regular haemodialysis. She complains of pain in her fingers. An X-ray shows digital subperiosteal erosions. What is the primary cause for her condition? Increased serum phosphate levels Increased parathyroid hormone levels

Decreased renal 1-hydroxylase enzyme levels Decreased serum calcium levels Increased serum alkaline phosphatase levels

Your answer

Decreased renal production of the 1 -hydroxylase enzyme results in reduced conversion of 25-dihydroxycholecalciferol to the more metabolically active 1,25dihydroxycholecalciferol. Receptors for this exist on the parathyroid glands, failure of occupancy of which leads to the increased release of parathyroid hormone. 1,25dihydroxycholecalciferol deficiency also results in gut calcium malabsorption. The other features are due to excess parathyroid hormone levels.

5. A 17-year-old young woman, who works in a crche, presents for review. She complains of joint pains, vomiting, diarrhoea and crampy abdominal pain. She also has a purpuric rash on her legs and over her belt line. She has microscopic haematuria, proteinuria and red blood cell casts on urine testing. What is the most likely underlying diagnosis in this case? Idiopathic thrombocytopenic purpura HenochSchnlein purpura (HSP) Your answer Polyarteritis nodosa Meningococcal septicaemia Thrombotic thrombocytopenic purpura HSP is the most common vasculitis seen in children and young adults. It is most commonly seen in the 415 year age group but may be seen in young adults. There is a 2:1 male to female ratio, and peak incidence occurs in the spring. Key physical findings include palpable purpura of dependent areas, especially the lower limbs and areas exposed to skin pressure. There may also be subcutaneous oedema, arthralgias in up to 80% of patients, GI symptoms in 30% and renal insufficiency in 1020% of patients. Anecdotal reports suggest that some cases may follow respiratory tract infection. Skin biopsy of lesions reveals a leukocytoclastic vasculitis. IgA levels are raised in approximately 50% of patients. Glomerulonephritis may be present and is identified by microscopic haematuria, proteinuria and red cell casts. Permanent renal insufficiency may unfortunately occur in up to 5% of patients. Prednisolone may be considered for severe disease, with the addition of azathioprine if glomerulonephritis is present and is associated with rapidly deteriorating renal function.

6. A 33-year-old man presents with rapid progressive glomerulonephritis and nephrotic syndrome. A renal biopsy shows Wegeners granulomatosis. What is the most appropriate treatment? Blood transfusion Cyclophosphamide and prednisolone Methotrexate Antibiotics Cyclosporin

Your answer

Focal necrotising glomerulonephritis is the characteristic renal lesion of generalised Wegeners granulomatosis and microscopic polyangiitis. Typically, it presents with deteriorating renal function that progresses to renal failure within three months, ie as rapidly progressive glomerulonephritis, although a few patients have disease that is more indolent. Proteinuria (typically 2 to 3 g/24 h, but occasionally in the nephrotic range) and microscopic haematuria provide clinical evidence of glomerulonephritis, as does urine microscopy that reveals granular and red cell casts. The combination of prednisolone and cyclophosphamide is now established as the standard induction therapy for patients with generalised Wegeners granulomatosis or microscopic polyangiitis. There is consensus on how corticosteroids should be used, but less so for cyclophosphamide. Prednisolone is given in doses of around 1 mg/kg/day initially, after which the dose is reduced rapidly, typically at weekly intervals. Controlled trials show that adding pulses of methyl prednisolone is unlikely to confer additional benefit.

7. The proximal convoluted tubule (PCT) is made up of a single layer of cells that interdigitate with one another and are united by apical tight junctions. It plays a major role in water and electrolyte metabolism. One of the most important functions of the PCT is: Bicarbonate secretion Glucose excretion Ammonia secretion Urine concentration

Sodium reabsorption Your answer Bicarbonate reabsorption is mediated at the proximal tubule level. Ammonia is secreted by the distal tubule. Regulation of water secretion is by the distal tubule and the collecting ducts under the influence of vasopressin. Sodium and glucose as well as amino acids are absorbed at the proximal tubule level.

8. A 78-year-old retired textile worker is noted to have haematuria on routine urinary dipstick at her GPs practice. Her only past history of note is arthritis, for which she takes anti-inflammatories. Plain abdominal X-ray reveals no evidence of renal stones. Routine bloods reveal increased plasma viscosity and mild anaemia, with a haemoglobin of 10.5 g/dl and a ferritin just below the normal range. What is the diagnosis that fits best with this clinical picture? Chronic urinary tract infection Bladder cancer Your answer Interstitial cystitis Analgesic nephropathy Interstitial nephritis Bladder cancer characteristically presents with painless haematuria, which may be gross, or microscopic haematuria detected on dipstick in early-stage disease. Later-stage disease may also precipitate abdominal pain or renal failure due to obstruction. Bladder cancer does have an increased association with cigarette smoking, occupational exposure to dyes or petroleum products and chemical exposure to nitrosamines. In the Western world around 93% of bladder cancers are transitional cell (TCC) in type, squamouscell carcinomas (SCCs) comprise around 6% of the total and adenocarcinomas less than 1%. At the time of diagnosis around 70% of carcinomas are still localised to the bladder, 20% extend to involve regional lymph nodes and 3% present with distant metastases. Some 80% of superficial bladder tumours are said to recur. Treatment of choice for localised tumours is transurethral tumour resection, with the use of intravesical chemotherapy where required.

9. A 47-year-old man attends the outpatient clinic complaining of swelling in the ankles and lethargy. On examination, his blood pressure is 160/90 and he is found to have pitting oedema in both legs. Laboratory investigations reveal:

Hb Urea Creatinine Hepatitis B antigen Anti-nuclear antibodies

11.5 g/dl 35 mmol/l 275 mol/l Positive Negative

What is the probable diagnosis? Membranous glomerulonephritis Your answer Hepatitis B infection Acute interstitial nephritis Renal tubular acidosis Systemic lupus erythematosus

Anti-nuclear antibodies screening is a good test for systemic lupus erythematosus (SLE) as more than 90% of patients have positive tests. SLE is more common in females and presents in more than two-thirds of patients with arthralgia, fever and skin lesions. Renal tubular acidosis presents with hypercalciuria, hyperphosphaturia, hyperchloraemia and hypokalaemia. Hepatitis B virus surface antigen does not cause acute interstitial nephritis. Membranous glomerulonephropathy is secondary in 2530% of cases and may be caused by hepatitis B virus. Most patients present with proteinuria.

10. You are called to a cardiac arrest in the Emergency Department where a patient who is known to the dialysis unit has been brought by ambulance. On reviewing her drugs you note that she is taking a statin, three antihypertensive agents, aspirin and has been receiving erythropoietin injections. Unfortunately resuscitation is unsuccessful. Which of the following is the commonest cause of death in renal dialysis patients? Gastrointestinal haemorrhage Occult malignancy

Pulmonary embolus Cardiovascular disease Overwhelming sepsis

Your answer

Patients undergoing haemodialysis have a number of risk factors associated with accelerated cardiovascular disease, which include low high-density lipoprotein (HDL) cholesterol, raised triglycerides and low-density lipoprotein (LDL) cholesterol levels due to chronic renal failure. More often than not they are hypertensive and require a number of antihypertensive medications to control blood pressure. They may have long standing vascular disease including renovascular disease, which precipitated the need for haemodialysis. Many patients have co-morbidities that put them at high risk of vascular disease such as diabetes mellitus. There is evidence that renal patients have chronically elevateds of C-reactive protein (CRP) and other inflammatory markers. Taken together, these multiple risk factors lead to a process of accelerated atherosclerosis.

11. A 56-year-old woman with a 15-year history of rheumatoid arthritis has been regularly taking diclofenac for pain relief. She presents with mild chronic renal failure, hyperkalaemia and acidosis. Blood tests show decreased plasma renin and aldosterone. What is the most probable diagnosis? Type-1 renal tubular acidosis Type-2 renal tubular acidosis Type-4 renal tubular acidosis Your answer Uraemic acidosis Acute tubulointerstitial nephritis

Type-4 renal tubular acidosis, also known as hyporeninaemic hypoaldosteronism, is the most common of the renal tubular disorders. The cardinal features are hyperkalaemia and acidosis occurring in a patient with mild chronic renal insufficiency, usually caused by tubulointerstitial disease or diabetes. An identical syndrome may be caused by the chronic ingestion of non-

steroidal anti-inflammatory drugs (NSAIDs), which impair renin and aldosterone secretion.

12. A 33-year-old woman attended her GP for new-patient screening and was found to be hypertensive, BP 155/90 mmHg. Early retinal changes consistent with hypertension are seen on fundoscopy. Following referral, an ultrasound scan reveals kidneys of broadly similar size within the normal range. Angiography reveals concentric narrowing of the left renal artery, with no evidence of atherosclerosis in the other areas of the vascular tree on imaging. Which of the following statements best fits the explanation, treatment and outcome for her condition? Her renal artery narrowing is likely to be progressive Her renal artery narrowing is unlikely to progress Your answer

Angioplasty is the treatment of choice for all patients with this clinical presentation She is likely to have atherosclerotic disease elsewhere Her hypertension is likely to be associated with reduced aldosterone secretion She fits the picture of a woman with fibromuscular hyperplasia of the renal artery. Renovascular hypertension is the cause of hypertension in 0.25% of all hypertensive patients. There may acute renal artery occlusion presenting with fever and flank pain, or a chronic presentation associated with occult hypertension, retinal changes consistent with hypertension, episodes of pulmonary oedema in older patients, mild hypokalaemia or renal failure following the initiation of angiotensin-converting enzyme (ACE)-inhibitor therapy. Fibromuscular hyperplasia may occur in young women commonly, whereas renal artery stenosis associated with atherosclerosis tends to occur in an older population group. Imaging may include a renal isotope scan, ultrasound to measure differential kidney size and angiography. Renal artery stenosis associated with fibromuscular hyperplasia does not progress, although BP control is clearly important to prevent associated complications. Atherosclerotic renal disease is progressive: in patients with > 60% stenosis, 5% progress to total occlusion by 1 year, 11% by year 2. Angioplasty may result in resolution of hypertension for some patients with fibromuscular hyperplasia.

13. A 22-year-old pregnant woman presents with dysuria and increased frequency. A urinary tract infection is diagnosed. What is the most appropriate step? No treatment

Should always be treated in pregnancy Treatment if the c-reactive protein (CRP) is high Treatment if the erythrocyte sedimentation rate (ESR) is high Treatment if recurrence

Your answer

Urinary tract infection is usually diagnosed by a bacterial count of >100 000/ml at MSU but significant infections can occur with lesser counts (pure growth of 1 organism): the infection is usually limited to the lower urinary tract and deterioration of renal function is not seen. Infections in pregnancy should be treated, as 25% of patients will develop acute pyelonephritis.

14. A 2-year-old boy presents with recurrent urinary tract infections. What is the most common cause for this problem in a child of this age? Posterior urethral valves Vesicoureteric reflux Neurogenic bladder Renal calculi Bilateral polycystic kidneys

Your answer

The most common abnormality seen in a child with urinary tract infection in a micturating cystourethrogram is vesicoureteric reflux. It is identified in approximately 40% of patients. The chief complaint of children with posterior urethral valves is a poor urinary stream. The other conditions occur later in life.

15. A 7-year-old boy presents with generalised oedema. Urinalysis shows marked albuminuria. Blood tests reveal hypoalbuminaemia and hyperlipidaemia. A renal biopsy appears normal on light microscopy. What would be the most characteristic finding on electron microscopy? Deposition of electron-dense material on the capillary basement membrane Splitting of the capillary basement membrane Fusion of foot processes of the glomerular epithelial cells Thinning of the capillary basement membrane Fibrils of amyloid protein in the mesangium

Your answer

The most common cause of nephrotic syndrome in children is minimal-change nephropathy. Loss of foot processes may be seen in other proteinuric states such as membranous glomerulonephritis and diabetic nephropathy, but light microscopic changes would also be evident in these conditions. Splitting of the capillary basement membrane is seen in mesangiocapillary glomerulonephritis, while thinning is noted in thin glomerular basement membrane disease.

16. A 19-year-old woman complains of stiff joints and a facial rash. Her blood pressure is 145/95 mmHg, she has +1 oedema, slightly swollen metacarpophalangeal joints and some ulceration of her buccal mucosa. Plasma creatinine is 92 mol/l, Hb is 8.9 g/dl and platelet count is 92 x 1012/l. Urinalysis shows +3 blood and +3 protein. High titres of double-stranded DNA antibodies are detectable in her serum. What pathogenic feature leads to the renal lesion? Arteriolar vasculitis Circulating antibodies directed against the glomerular basement membrane Deposition of a linear dense deposit within the glomerular basement membrane Subendothelial deposition of immune complexes Subepithelial deposition of immune complexes The type of renal disease that develops in systemic lupus erythematosus (SLE) is dependent on the site of deposition of the immune deposit, which may in turn be dependent on the site of immune complex formation. Circulating immune complexes, which tend to be large and anionic, cannot cross the glomerular basement membrane and thus become deposited between the vascular endothelium and the GBM (subendothelial). These complexes are efficient at

Your answer

fixing complement, thereby generating chemoattractants such as C3a and C5a, which have direct access to the circulation and result in the influx of immune effector cells such as neutrophils and monocytes. These changes are manifested histologically by mesangial and proliferative glomerulonephritis and clinically by the nephritic syndrome, with haematuria being prominent. On the other hand, immune complexes that are formed in situ, or that are cationic, tend to deposit between the GBM and the epithelium of Bowmans space (subepithelial). These are also capable of activating complement, but the presence of the GBM between them and the circulation prevents the free diffusion of chemoattractants and influx of circulating effector cells. Thus, the urine sediment is bland, with little or no haematuria, although proteinuria may be massive. This is manifested histologically by membranous nephropathy. 17. A 24-year-old patient is admitted after falling asleep drunk in a toilet cubicle. On examination his BP 130/70, pulse 98 bpm and there is no pericardial rub. There is boggy tenderness of his right calf and both thigh muscles. Biochemistry reveals urea 38 mmol/l, creatinine 410 mol/l, bicarbonate 15 mmol/l, pH 7.1, potassium 7.8 mmol/l and creatine kinase 17 000 IU/l. What is the most appropriate form of treatment? Continuous ambulatory peritoneal dialysis Dopamine Forced alkaline diuresis Haemodialysis High-dose diuretic therapy

Your answer

This patient has rhabdomyolysis, which is suggested by the raised creatine kinase. The resulting renal failure is due to the toxic effects of myoglobin upon the renal tubular cells. This patient will probably require fasciotomy, so continuous therapies that are slower and require anticoagulation are not ideal. Rapid correction of the life-threatening hyperkalaemia and acidosis is necessary and intermittent haemodialysis is the treatment of choice. Forced alkaline diuresis may prevent acute renal failure, but once renal failure is established with life-threatening hyperkalaemia, it is inappropriate.

18. A 56-year-old man presents with flank pain and haematuria. His plasma creatinine concentration is 368 mol/l and renal ultrasound reveals bilateral 14-cm kidneys with multiple cysts. There are also cysts scattered through the liver. What is the cause of this condition? Bilateral ureteric reflux from childhood Genetic defect in the gene encoding polycystin Genetic defect in the von HippelLandau gene Genetic defect in the gene encoding type-IV collagen Genetic defect in the gene encoding cystinosin

Your answer

Some 85% of cases of adult polycystic kidney disease (ADPKD) are associated with a mutation in the PKD1 gene, which results in the absence of functioning polycystin. This novel protein has unknown functions but may operate as a cell-membrane ion transporter. Most of the remaining cases of ADPKD are associated with a defect in PKD2, the transcriptional effects of which are unknown. Mutations in PKD1 tend to result in a more severe phenotype with end-stage renal failure (ESRF) typically occurring in the fifth to sixth decade of life, whereas patients with a PKD2 mutation tend to develop renal failure in the eighth decade of life. The von HippelLandau gene is a tumour-suppressor gene on chromosome 3, defects in which result in von HippelLandau syndrome. Patients with Alports syndrome (a condition not associated with renal cysts) have mutations in the -5 chain of type-IV collagen and cystinosin is a lysosomal membrane transport protein that is defective in cystinosi

19. A 55-year-old lady with chronic renal failure presents with numbness and tingling in the lower limbs. Earlier in the day, she temporarily lost consciousness and was told that she had fits during that period. There is loss of sensation to pinpricks below both knees. Blood tests revealed a haemoglobin of 5.8 g/dl and a serum creatinine level of 540 mol/l. She requires: Urgent blood transfusion Electroencephalography Carbamazepine therapy Renal dialysis Your answer Sodium bicarbonate supplements The symptoms are due to uraemia. The presence of uraemic peripheral neuropathy is an indication for dialysis. Early dialysis may reverse the sensory deficits. You would avoid blood transfusion before haemodialysis due to the risk of fluid overload and subsequent decompensation.

20. Renal tubular acidosis (RTA) results from either a defect in reabsorption of bicarbonate in the proximal tubule or a failure of acidification of the urine in the distal tubule. What is the most significant clinical presentation of distal renal

tubular acidosis (type I) that differentiates it from proximal renal tubular acidosis (type II)? There is failure to thrive Renal stone formation Urinary pH in Type I can be lowered below pH 5.3 after ammonium chloride administration Metabolic acidosis is common Potassium depletion occurs

Your answer

Normal anion gap, growth failure, hyperventilation and systemic acidosis are features of both types. Type Irenal tubular acidosis is severe and is due to failure of the collecting ducts to secrete hydrogen ions or to sustain the gradient for hydrogen ions between the luminal fluid and the tubular cell. The urine pH will not lower below 5.3 even with maximal acid load. Nephrocalcinosis is a feature of type I. Type II is due to bicarbonate loss from the tubules and is commonly part of syndromes like Fanconi's syndrome or Wilson's disease.

21. A 40-year-old man with hypertension presents with frank haematuria. He gives a history that his uncle recently underwent a renal transplant and that his father died of renal failure. On physical examination, a large mass is felt over the right lumbar area. A smaller mass is felt in the left flank. Blood urea and serum creatinine levels are raised. What is the most probable diagnosis? Renal cell carcinoma Adult polycystic kidney disease Your answer Renal calculi Prostatic carcinoma Renal amyloidosis Adult polycystic kidney disease is an autosomal-dominant condition. It is mostly bilateral and presents with acute loin pain and/or haematuria. There is a gradual decline in renal function and is a common cause of chronic renal failure. Hypertension is an early and very common feature of this condition.

The average age of presentation of renal cell carcinoma is around 55 years. Patients present with a classic triad of haematuria, loin pain and a unilateral mass in the flank. Malaise, anorexia and weight loss may occur and one-third of patients has hypertension. Pain in ureteric calculus, which is most often associated with haematuria, is extremely severe. Prostatic carcinoma presents with symptoms of lower urinary tract obstruction or of metastatic spread, particularly to bone. The cancer usually appears in older men over the age of 60 years. Renal amyloidosis presents with asymptomatic proteinuria, nephritic syndrome or renal failure. Frank haematuria is not a feature.

22. A 65-year-old woman with longstanding diabetes presents with proteinuria. Her serum creatinine level is normal. What would be the most common renal complication in this case? Renal papillary necrosis Renal infarction Chronic pyelonephritis Glomerulosclerosis Obstructive uropathy

Your answer

Diabetics are prone to atherosclerosis, urinary infection and papillary necrosis but glomerular lesions cause most of the problems. Typically, there is thickening of the glomerular basement membrane and glomerulosclerosis that may be diffuse or nodular (KimmelstielWilson disease).

23. A 35-year-old man presents with cough, haemoptysis and glomerulonephritis. His cANCA [antimyeloperoxidase] levels are elevated. What is the most likely cause for these features? Goodpastures syndrome Polyarteritis nodosa Wegeners granulomatosis Kawasakis disease Microscopic polyangiitis

Your answer

Both Wegeners granulomatosis (WG) and Goodpastures syndrome can present with these clinical features, but the presence of elevated levels of c-ANCA (cytoplasmic-

antineutrophilic cytoplasmic antibodies) is in favour of the former. Almost all patients with active systemic WG have positive ANCA findings, commonly directed against serine proteinase 3. Approximately 80-95% of all ANCA found in patients with WG is cANCA/antiserine proteinase 3. Most of the remaining 5-20% isp-ANCApositive with antibodies directed against myeloperoxidase. The diagnostic accuracy of ANCA testing becomes greater in patients with the classic presentation, in whom c-ANCA has a 98% post-test probability of predicting the diagnosis WG. In Goodpastures syndrome, polyarteritis nodosa and microscopic polyangiitis, p-ANCA is elevated. Kawasakis disease is an acute systemic vasculitis mainly affecting children under 5 years of age. The clinical features are fever lasting for 5 days or more, bilateral conjunctival congestion, dryness and redness of the lips and oral cavity 3 days after onset, acute cervical lymphadenopathy, polymorphic rash and redness and oedema of the palms and soles 25 days after onset.

24. A 14-year-old boy presents with hypertension and acute renal failure after an episode of diarrhoea. What is the most likely diagnosis? Haemolyticuraemic syndrome IgA nephropathy HIV Legionellosis Salmonellosis Your answer

Haemolyticuraemic syndrome is characterised by a microangiopathic haemolytic anaemia, associated with hypertension and acute renal failure. It may be found in an epidemic (diarrhoea-positive) form in association with acute gastroenteritis (eg due to verotoxin producing strains of Escherichia Coli), or it may occur in a sporadic (diarrhoeanegative) form. In some patients there appear to be inhibitors of prostaglandin production within the patients serum. The prognosis is usually better in children and in those with the diarrhoeaassociated epidemic form. Although it may be a self-limiting process requiring supportive treatment alone, various specific treatments have been tried including infusions of freshfrozen plasma (FFP), plasma exchange, prostaglandin infusions and high-dose vitamin E. The majority of patients have almost complete return of renal function following recovery. 25. Which one of the following conditions is most commonly associated with large kidneys despite advanced renal failure? Amyloidosis Your answer

Hypertensive nephrosclerosis Membranous glomerulonephritis Systemic sclerosis Analgesic nephropathy

Other causes include: Stage 1 diabetic nephropathy Hydronephrosis Acromegaly Renal vein thrombosis

26. A 15-year-old girl presents with red spots on her buttocks and legs, joint pains, oedema, hypertension and proteinuria. She had an upper respiratory tract infection 23 weeks ago. What is the most likely diagnosis? Proliferative glomerulonephritis Goodpastures syndrome Wegeners granulomatosis HenochSchnlein purpura Systemic lupus erythematosus

Your answer

This girl most probably has HenochSchnlein purpura. It presents with a purpuric rash over the buttocks and extensor surfaces. Some one-third of patients has nephritis. Joint pains and acute abdominal pain are common. The fault lies in the vasculature; the platelets are normal. It often follows an acute respiratory tract infection and usually follows a benign course over weeks or months. Complications include massive gastrointestinal haemorrhage, ileus and, rarely, renal failure.

Wegeners granulomatosis would have additional features such as nasal ulceration, rhinitis, otitis media and pulmonary symptoms. Variable shadows may be seen on chest X-ray, particularly multiple nodules. Lung involvement (pulmonary haemorrhage) is seen in Goodpastures syndrome: the chest X-ray may show diffuse infiltrates in the lower zones. Renal involvement in systemic lupus erythematosus occurs in 50% of patients but is seldom so rapidly progressive as to cause oedema, hypertension and proteinuria in 23 weeks time. Proliferative glomerulonephritis would present with macroscopic haematuria, but purpura is not a feature of this condition.

27. A 67-year-old man with type-2 diabetes had a serum creatinine concentration of 250 mol/l (60110) before admission for radiographic investigation including intravenous contrast medium visualisation. He is admitted to A&E 2 days after discharge home. His creatinine concentration is now 470 mol/l and he has only passed 20 ml of urine since being catheterised some 3 h ago. You suspect contrast nephropathy. Which of the following best describes the incidence of contrast nephropathy in such a patient? 5% 70% 8% 25% Your answer 50% In an unselected population the incidence of contrast nephropathy is said to be between 2 and 7%. If renal function is impaired then the incidence increases to around 25%. In those patients with pre-existing renal disease without diabetes the incidence of contrast nephropathy is thought to be around 60% when the creatinine concentration is above 400 mmol/l. If these patients have diabetes mellitus as well, then the incidence of acute renal failure is said to be 100%. Congestive cardiac failure carries a risk of contrast nephropathy of 78%. Other risk factors of note include multiple myeloma, pancreatitis, multiple contrast studies, dehydration and jaundice. Outcome figures from one study suggested that recovery of renal function occurs in 68% of patients, partial recovery in 14%, with death, dialysis or transplantation occurring in 18%. 28. A 45-year-old woman presents with increased frequency of micturition, dysuria and suprapubic pain 5 days after completing antibacterial treatment for an E. coli urinary tract infection. She is also using a barrier contraceptive. Urine microscopy shows the presence of E. coli. What is the most likely cause for her problem? Atrophic vaginitis Use of spermicidal jelly

Renal stones Your answer Interstitial cystitis Chlamydial urethritis This patient has had a relapse. Relapse is diagnosed by the recurrence of bacteriuria with the same organism within 7 days of completing antibacterial treatment, and implies failure to eradicate infection. It usually occurs in conditions in which it is difficult to eradicate the bacteria, such as stones, scarred kidneys, polycystic disease or bacterial prostatitis. Reinfection occurs when bacteriuria is absent after treatment for at least 14 days, usually longer, followed by recurrence of infection with the same or a different organism. Interstitial cystitis commonly affects women over the age of 40 years. It presents with frequency, dysuria and often severe suprapubic pain. Urine cultures are sterile.

29. A 35-year-old woman with a past medical history of peptic ulceration presents with a 3day history of vomiting. Investigations reveal: haemoglobin 12.2 g/dl, sodium 130 mmol/l, potassium 3.0 mmol/l, urea 14 mmol/l, bicarbonate 34 mmol/l, chloride 85 mmol/l and pH 7.52. What treatment would be most appropriate in this condition? IV 5% dextrose IV normal saline with potassium supplementation Acetazolamide Sodium bicarbonate Thiazide diuretics

Your answer

This patient has hypochloraemic alkalosis and hypokalaemia. Treatment with oral potassium supplements is sufficient in this case. Isotonic saline alone will not correct the potassium deficiency nor will acetazolamide, which causes bicarbonate diuresis. Sodium bicarbonate and thiazide diuretics are useful in renal tubular acidosis. 30. A 4 year old girl is suspected to have vesicoureteric reflux as she has had a number of urinary tract infections. Which investigation would be most useful in this case? Plain X-ray of the abdomen Excretion urography Computed tomography Micturating cystourethrography Retrograde pyelography

Your answer

A micturating cystourethrography is used primarily in children to check for vesicoureteric reflux during voiding. It is not useful in adult women because by this time the reflux tends to disappear. Retrograde pyelography is mainly used to investigate lesions of the ureter and to define the lower level of ureteral obstruction shown on excretion urography or ultrasound. The other investigations are only useful for upper urinary tract disorders.

31. An 8-year-old boy complains of nausea and vomiting and fatigue. On examination, he is oedematous and his blood pressure is 160/100 mmHg. Blood urea and serum creatinine levels are normal. Urine microscopy shows oval fat bodies. 24-hour urine protein is 1.5 g. The doctor plans to start a course of corticosteroids, which he says will cure the condition. What is the most probable diagnosis? Proliferative glomerulonephritis Membranous glomerulonephritis Minimal-change disease Acute tubular necrosis Amyloidosis of the kidneys

Your answer

Minimal-change disease (MCD), also known as lipoid nephrosis or nil disease, is the most common single form of nephrotic syndrome in children. It refers to a histopathologic lesion in the glomerulus that almost always is associated with nephrotic syndrome. It typically is a disease of childhood, but it also can occur in adults. Corticosteroids are the treatment of choice, leading to complete remission of proteinuria in the majority of cases. Amyloidosis is usually seen in middle-aged patients (ie those over 40 years of age). Steroid therapy is of no proven value in acute tubular necrosis. 32. A young man presents with haematuria and recurrent haemoptysis. Renal biopsy shows the presence of antiglomerular basement membrane antibody. What is the most characteristic radiological finding on chest X-ray in this case? Bilateral hilar lymphadenopathy Lobar consolidation Patchy shadows in apical zones Bilateral diffuse infiltrates in the lower zones Pleural effusion

Your answer

The diagnosis is Goodpastures syndrome. A chest X-ray would show bilateral diffuse infiltrates often in the lower zones. This represents areas of pulmonary haemorrhage. Bilateral hilar lymphadenopathy is a characteristic feature of sarcoidosis. Lobar consolidation is seen in pneumonias, while patchy shadows in apical zones are typical of tuberculosis. Pleural effusion does not occur in this condition.

33. A 60-year-old man is undergoing investigation for suspected nephrotic syndrome. What would the results of a blood test be most likely to show? Increased antithrombin-III levels Decreased fibrinogen levels Decreased thyroxine levels Normal folate levels Raised blood glucose levels

Your answer

Thyroxine levels are depressed in nephrotic syndrome due to the enhanced urinary excretion of thyroxine-binding globulin. There is associated hypercoagulability due to urinary loss of antithrombin-III, proteins C and S and a concomitant increase in fibrinogen levels. Renal vein thrombosis occurs in 1520% of patients with nephrotic syndrome. Folate levels are usually unaffected. Raised blood glucose indicates diabetes mellitus.

34. A 72-year-old woman presents acutely unwell to casualty. She has been suffering from gastroenteritis for a few days. Past medical history of note includes furosemide and ramipril for mild heart failure and treatment for nose bleeds. A full blood count and film reveals evidence of red cell haemolysis and thrombocytopenia, serum creatinine is 290 mol/l and urea is 17.0 mmol/l. What diagnosis fits best with this clinical picture? Acute dehydration Haemolyticuraemic syndrome (HUS) Your answer Wegeners granulomatosis Furosemide toxicity Renal artery stenosis HUS is associated with Escherichia coli infection, particularly E. coli 157. There is intravascular haemolysis, thrombocytopenia, and acute renal failure. It has been suggested that acute infection triggers endothelial damage in susceptible individuals. Inherited recurrent HUS has been described with both dominant and recessive patterns of inheritance. Most children recover spontaneously from the illness, but mortality may be high in the elderly. Treatment with heparin, fresh-frozen plasma, prostacyclins and platelet inhibitors have all been tried, but there have been no controlled clinical trials to date. Some commentators propose that antibiotic treatment of E. coli may actually have increased the risk of HUS.

35. A 23-year-old man presents to his GP with rapidly increasing shortness of breath and haemoptysis for the second time within 3 weeks. He is referred urgently to the medical oncall team. Apart from smoking 20 cigarettes per day and having mild asthma, he has no other significant past medical history. Urinalysis reveals microscopic haematuria and proteinuria. Chest X-ray reveals fluffy pulmonary infiltrates. Haemoglobin is 10.5 g/dl, serum creatinine is 345 mol/l, c-ANCA is negative. What diagnosis fits best with this clinical picture? Bronchopneumonia Goodpastures syndrome Your answer Wegeners granulomatosis SLE Sarcoidosis Goodpastures syndrome is characterised by the triad of glomerulonephritis, pulmonary haemorrhage and anti-GBM antibody. The negative c-ANCA (result makes Wegeners granulomatosis (one possible differential) much less likely. Goodpastures syndrome predominantly affects young male smokers, the male:female ratio being 6:1, and Goodpastures syndrome accounts for 5% of all cases of rapidly progressive glomerulonephritis. Some 80% of patients with Goodpastures syndrome are HLA-BR2 positive. Acute management may involve treatment with prednisolone and cyclophosphamide or plasma-exchange therapy. In patients with severe renal failure, haemodialysis may be required

36. A 21-year-old man presents to A&E for the third time with recurrent urinary stones. There appear to be no predisposing factors and he is otherwise well, urine culture is unremarkable. Slightly unusually, these stones turn out to be cystine stones. What is the most likely diagnosis in this case? Cystinosis Cystinuria Your answer Familial iminoglycinuria Fanconis syndrome Hartnups disease Cystinuria is related to the defective tubular reabsorption and jejunal absorption of cystine and the other dibasic amino acids lysine, ornithine and arginine. Inheritance is recessive or incompletely recessive, some heterozygotes may only have an increased excretion of lysine and cystine. Although jejunal cystine absorption is impaired, some absorption of cystine in peptide form may occur. In total, cystinuria is responsible for around 12% of all urinary calculi. Patients are encouraged to maintain a high fluid intake, at above 3 litres per day, including drinking fluids at night. Penicillamine may be used for those patients with

recurrent stones who are unable to keep their urinary cystine concentration low. Cystinosis occurs due to lysosomal cystine accumulation, the nephropathic form presenting in the first year of life with failure to thrive. Cystine deposits in the retinas and corneas occur, leading to progressive renal impairment. A less aggressive, non-nephropathic form presents in young adults.

37. A 71-year-old diabetic woman is in her 6th year of haemodialysis. She visits her GP with symptoms of pain, numbness and tingling in both hands during the early hours of the morning. She also complains of stiffness in her shoulders, hips and knees. What diagnosis fits best with this clinical picture? Rheumatoid arthritis Dialysis amyloidosis Your answer Diabetic neuropathy Seronegative arthritis Uraemic neuropathy Dialysis amyloidosis occurs due to the failure of clearance of 2-microglobulin. This protein, the light chain of class-1 HLA antigens, is usually freely filtered at the glomerulus but is not cleared by cellulose-based dialysis membranes. There is resulting amyloid deposition within the synovium, which appears to result in a clinical syndrome of median nerve compression and pain and stiffness in multiple joints. The syndrome resolves slowly after renal transplant, and some benefit is seen in switching patients to dialysis with a biosynthetic dialysis membrane. There may be associated GI haemorrhage caused by amyloid deposition around submucosal blood vessels.

38. An 18-year-old man is recovering from meningococcal septicaemia, treated with highdose cefotaxime. He suffered gangrene in four toes, which necessitated amputation. Postoperatively, he complained of flank pain and is now becoming drowsy and lethargic. His blood pressure is 90/45 mmHg and his peripheries are cool. Plasma creatinine is 156 mol/l, urea is 29.6 mmol/l, potassium is 6.7 mmol/l, sodium is 119 mmol/l and bicarbonate is 17 mmol/l. Spot urine Na+ is 136 mmol/l. What is the likely cause of his deterioration? Acute tubular necrosis Acute interstitial nephritis secondary to cefotaxime Adrenal insufficiency Septic shock

Your answer

SIADH Bilateral adrenal haemorrhage may occur in the setting of septicaemia. This was initially described in cases of meningococcaemia with disseminated intravascular coagulation (DIC) (WaterhouseFriderichsen syndrome), although in a recent series pseudomonas infection was the commonest cause. Adrenal insufficiency results in mineralocorticoid deficiency and potentially life-threatening salt-wasting and a failure to excrete potassium. The combination of hyperkalaemia, hyponatraemia and metabolic acidosis should always suggest hypoadrenalism.

39. A 62-year-old man with a long-standing history of hypertension is seen in the outpatient clinic. Investigations gave:

Creatinine 280 mmol/l Urinalysis, blood++ Protein 1.8 g/l Ultrasound scan of kidneys showed left kidney 8.5 cm and right kidney 8.9 cm

What is the best investigation to diagnose the cause of his renal impairment? Intravenous urogram (IVU) Isotope renogram Renal angiogram Renal biopsy Retrograde pyelogram

Your answer

This man has glomerulonephritis, as demonstrated by the presence of proteinuria and haematuria, and the best way to establish the underlying diagnosis is via renal biopsy. There is a long history of hypertension and deranged renal function now, which is compatible with a possible diagnosis of membranous glomerulonephritis. Basement membrane thickening caused by immune complex deposition is the main feature of the disease. Associations include systemic lupus erythematosus (SLE), bowel and bronchus malignancy, penicillamine therapy, and hepatitis B infection. A significant number of cases are idiopathic. There is a preponderance of male cases, and presentation is most common in adulthood. Approximately one-third of patients develop renal failure within 1020 years of diagnosis. Patients with heavy proteinuria and progressive renal impairment may benefit from immunosuppressive therapy.

40. While managing the serum potassium levels in a young man with acute renal failure following a road traffic accident, what is the most important biochemical factor that should be kept in mind? Acidosis leads to the movement of potassium from the extracellular to the intracellular fluid compartment Tissue destruction or breakdown results in the release of intracellular Your potassium answer Potassium release from muscle cells leads to gluconeogenesis Adrenaline inhibits the entry of potassium into cells, thus increasing the plasma potassium level There is a direct relationship between potassium levels and blood pH Potassium is the main intracellular cation. Acidosis therefore leads to the movement of potassium from the intracellular to the extracellular fluid compartment. Potassium release from muscle cells leads to glycogenolysis. Adrenaline stimulates the entry of potassium into cells, thus decreasing the plasma potassium level. There is an inverse relationship between potassium levels and blood pH. Thus hypokalaemia occurs during alkalosis, while hyperkalaemia is seen in metabolic acidosis.

41. You are reviewing a 46-year-old woman who is suffering from nephrotic syndrome secondary to the long-term use of penicillamine for rheumatoid arthritis. You note she has a raised erythrocyte sedimentation rate (ESR) and arrange for serum protein electrophoresis as part of a routine blood screen. Which of the following stems best fits the abnormalities that are typically seen on protein electrophoresis in patients with nephrotic syndrome? Increased serum albumin concentration Increased 1-globulin fraction Increased 1- and 2-globulin fractions, increased serum albumin Increased 1- and 2-globulin fractions, decreased serum albumin Your answer Increased 2-globulin fraction, decreased serum albumin In the nephrotic patient, abnormal findings on serum electrophoresis include a decreased albumin level and an increase in the 2-globulin fraction. A monoclonal paraprotein band will also be present where myeloma is the underlying cause, and there may be associated immune paresis with reduced concentrations of one or more of the immunoglobulins IgG, IgA or IgM. Some 10% of patients with myeloma will also have renal amyloid deposition. Possibly related to immunoglobulins lost in the urine, sepsis is an important complication of nephrotic syndrome. Infection with Pneumococcus spp is relatively common in affected individuals, and vaccination should always be offered. Early recognition of sepsis and

aggressive treatment is the key to a positive outcome.

42. You are reviewing a 78-year-old woman who has been admitted in a dehydrated and confused state to the Emergency department. Her bloods are markedly abnormal: potassium of 7.2 mmol/l (3.54.9), creatinine 450 mol/l (60110), urea 31.2 mmol/l (2.5 7.5). You repeat the serum potassium and it is still markedly raised. What is the most appropriate initial management in this case? Arrange continuous ECG monitoring and consider giving 10 ml of 10% Your calcium gluconate iv answer Arrange an urgent renal ultrasound scan Give intravenous salbutamol therapy Give 250 mg furosemide iv to reduce the potassium concentration Consider a third blood sample to confirm hyperkalaemia Once true hyperkalaemia is confirmed via a repeat blood sample, the management pathway is clear. Continuous ECG monitoring is a prerequisite. Intravenous administration of 10 ml 10% calcium gluconate should be considered for cardioprotection. Because insulin drives potassium ions from the extracellular to the intracellular compartment, 50 ml of 50% dextrose containing 10 U of insulin should be considered as the next intervention. The b2-agonist salbutamol also drives potassium into the intracellular compartment, but it would carry a significant risk of arrhythmia if given intravenously; nebulised salbutamol may also have positive effects in reducing serum potassium. Correction of severe acidosis may also redress some hyperkalaemia, but may exacerbate fluid retention in patients in renal failure. Other management for this particular patient is likely to include central venous pressure monitoring, catheterisation for close monitoring of fluid balance and an urgent ultrasound scan to determine the underlying cause of her renal dysfunction. Although furosemide may be of use to drive renal potassium excretion, it should be reserved until the results of her fluid balance assessment and ultrasound scan are known. Renal replacement therapy may be considered as the final option. However, it is important that an assessment of likely prognosis is determined first, ie because of the difficulties of withdrawing inappropriate treatment once it has been commenced.

43. A 50-year-old woman develops haematuria with exquisite right loin pain. She is restless and is unable to find a comfortable position that would ease the pain. On examination, her abdomen is soft with tenderness over the right lumbar region. She is afebrile. What is the most probable diagnosis?

Acute pyelonephritis Renal cell carcinoma Polycystic kidney disease Renal calculi Acute nephritic syndrome

Your answer

A ureteric calculus will cause severe loin pain. The absence of fever rules out acute pyelonephritis. Excruciating loin pain is not a feature of acute glomerulonephritis. Polycystic kidney disease may present with loin pain only if there is nephrolithiasis. Renal cell carcinoma may present with haematuria, loin pain and a mass in the flank. The pain, however, is not so severe. Pyrexia is present in about one-fifth of all patients.

44. A patient undergoes investigation for chronic upper urinary tract obstruction with intravenous urography. The patient should be informed about which severe complication? Contrast hypersensitivity Ureter damage Skin reaction Bleeding Development of renal stones Your answer

Intravenous urography will usually demonstrate the site, cause, and degree of obstruction and is much less operator dependent than ultrasound since the number of images checked by the clinician is equal to the number of images reported by the radiologist. Its major disadvantage is that the technique carries a mortality owing to contrast hypersensitivity reaction of perhaps 1 in 200 000. It also involves an intravenous injection, exposure to radiation (of particular concern in pregnant women and children), worsening of pain due to the diuretic effect of contrast medium when an upper tract (or tracts) is obstructed, and the potential for contrast nephrotoxicity. Patients with impaired renal function, particularly those with diabetes and perhaps patients with myelomatosis, are at particular risk. Such risk is minimised by employment of low osmolality contrast medium and the avoidance of prior dehydration.

45. A patient with chronic pain relieved by phenacetin (a NSAID) presents with progressive renal failure due to analgesic nephropathy. What is the most significant complication?

Urinary tract malignancy Bone tumours Liver fibrosis Pancreatitis Diabetes mellitus

Your answer

The renal manifestations of analgesic nephropathy are usually non-specific: normal renal function or slowly progressive chronic renal failure, and urinalysis that may be normal or may reveal sterile pyuria and mild proteinuria (less than 1.5 g/day). Hypertension and anaemia are commonly seen with moderate to advanced disease. More prominent proteinuria that can exceed 3.5 g/day can also occur at this time, a probable reflection of secondary haemodynamically mediated glomerular injury. Urinary tract malignancy will develop in as many as 810% of patients with analgesic nephropathy, but in well under 1% of phenacetin (a NSAID)-containing analgesic users without kidney disease. In women under the age of 50, for example, analgesic abuse is the most common cause of bladder cancer, an otherwise unusual disorder in young women.

46. A 65-year-old diabetic man presents with ascites and proteinuria. A renal biopsy shows mesangial deposits of monoclonal kappa and lambda light chains. What is the most probable diagnosis? Mesangioproliferative glomerulonephritis Focal glomerulosclerosis KimmelstielWilson disease Amyloidosis Multiple myeloma

Your answer

Amyloid deposition in cases of primary renal amyloidosis is typically seen within the mesangium. Amyloid light chain (AL amyloid) proteins are deposited. This is made up of kappa and lambda chains. In mesangioproliferative glomerulonephritis, IgG, IgA and C3 deposits are seen in the mesangium. In focal glomerulosclerosis, hyaline deposits consisting of IgM, C3, IgA and fibrinogen are deposited in juxtamedullary capillaries. KimmelstielWilson disease is characterised by hyaline masses in the mesangial core of the glomerular lobules. These masses consist of lipids and fibrin. In multiple myeloma, free kappa or lambda light chains are excreted. Blockage of tubules by casts accounts for the proteinuria and chronic renal failure.

47. A 36-year-old man with a history of intravenous drug use is found to have dipstick-positive haematuria. His blood pressure is 170/90 mmHg, he appears clinically well and he has a trace of peripheral oedema. Plasma creatinine is 140 mol/l, bilirubin is 65 mol/l, AST is 78 IU/l and his 24-hour urinary protein excretion rate is 4.1 g/24 h. Microscopy of the spun urine sediment reveals the presence of red-cell casts. Complement C3 is 0.5 (0.71.3) and C4 is 0.09 (0.12 0.27). What is the aetiology of the renal abnormalities? Hepatorenal syndrome Infection with hepatitis C Your answer Infection with HIV Infectious endocarditis Renal emboli Hepatitis C is now considered the principal cause of idiopathic mesangiocapillary glomerulonephritis, an immune-complex deposition disorder that presents with a mixed nephriticnephrotic picture associated with panhypocomplementaemia. Hepatitis C is endemic among the intravenous drug-using population. Other chronic sources of immune complexes, such as hepatitis B, schistosomiasis, chronic abscess, malaria and leprosy, may also be associated with MCGN. Infectious endocarditis is the principal differential diagnosis in this case and may present with haematuria and hypocomplementaemia, although hypertension is unusual and other manifestations of infection, such as fever, would usually be present. HIV nephropathy is suggested by the presence of nephrotic syndrome, rapidly progressive renal impairment and large kidneys on ultrasound. Renal emboli tend to present with flank pain and haematuria, whereas hepatorenal syndrome occurs in patients with advanced cirrhosis with marked peripheral oedema and cirrhosis.

48. A 52-year-old woman has been visiting her GP for a while, complaining of recurrent nosebleeds. She has also consulted for cosmetic surgery advice about the shape of her nose as it seems to be dipping, and presents for examination under anaesthetic to a private surgeon. Preoperative blood testing reveals anaemia with Hb of 10.5 g/dl (11.515.5), and

urinalysis reveals haematuria, red blood cell casts and proteinuria. Her surgery is cancelled, and further testing reveals a serum creatinine concentration of 195 m mol/l (60110). What is the most likely diagnosis in this case? ChurgStrauss syndrome Sarcoidosis Goodpastures syndrome Recurrent bacterial sinusitis Wegeners granulomatosis Your answer The mean onset of Wegeners granulomatosis is 40 years of age, with an incidence of 0.5/100,000 individuals. Classically, it is said to consist of necrotising granulomatous lesions in the upper or lower respiratory tracts, a focal necrotising vasculitis involving both arteries and veins and a focal glomerulonephritis of the kidneys. Individual manifestations with differing severity in certain organ systems may occur. Laboratory screening involves testing of cANCA (cytoplasmic antineutrophil cytoplasmic antibody). Patients are often anaemic with a leucocytosis and there are urine changes similar to those described above, which represent a glomerulonephritis-type picture. Lung involvement causes chest X-ray changes, these may be bilateral multiple nodules, cavitated mass lesions or pleural effusion in 20% of cases. Pulmonary function testing may reveal a restrictive lung defect. Treatment of choice is cyclophosphamide and steroid therapy. The 5-year survival with aggressive treatment is around 80%.

49. A 30-year-old man is found to have macroscopic haematuria, proteinuria of 1.5 g/24 hours and a serum creatinine level of 153 mol/l. What could be the possible diagnosis? Diabetic nephropathy Membranous glomerulonephritis Henoch-Schnlein purpura Focal glomerulosclerosis IgA nephropathy Your answer

Macroscopic haematuria is uncommon in membranous glomerulonephritis and diabetic nephropathy. Proteinuria would also be much greater (> 3.5 g/24 hours) in these conditions and in focal glomerulosclerosis. Henoch-Schnlein purpura would be associated with a petechial rash and systemic vasculitis. IgA nephropathy presents with hypertension, haematuria and proteinuria which may be exacerbated in the presence of respiratory infections.

50. An 85-year-old woman complains of dysuria and increased frequency of micturition. Urinalysis shows microscopic haematuria and 23 white cells per high power field. The urine culture is sterile. What treatment would be most appropriate in her case? Oral prednisolone Topical corticosteroid cream Alkalinisation of the urine Broad-spectrum antibiotic therapy Topical oestrogen cream

Your answer

This postmenopausal woman has symptoms caused by the urethral syndrome. The condition is common in elderly women due to dryness and atrophy of the urethral tissue. Topical oestrogen cream often has a dramatic response. As there is no infection, antibiotic therapy or alkalinisation of the urine will be of no benefit. Corticosteroids will again not be helpful.